Rituximab treatment for thrombotic thrombocytopenic purpura (TTP)

This leaflet is to inform patients and their carers about rituximab treatment for thrombotic thrombocytopenic purpura (TTP), why and how it is given, and what to expect during and after treatment.

What is thrombotic thrombocytopenic purpura?

Normally, your immune system produces antibodies and immune cells to fight invading viruses and bacteria. It is your body’s defence against infection and illness. But if the immune system malfunctions, as in autoimmune disease, it fails to recognise healthy cells, tissues and organs and, instead, attacks them.

In acquired autoimmune-mediated TTP (iTTP), patients develop antibodies against the enzyme called ADAMTS13. Lack of this enzyme cause blood clots forming spontaneously throughout the body (thrombosis). When these clots are formed, platelets are used up which leads to low circulating platelet in the blood (thrombocytopaenia). This causes bleeding underneath the skin (purpura). Hence the name thrombotic thrombocytopenic purpura.

About rituximab

Rituximab is made up of synthetic protein antibodies which behave like natural ones in the immune system. They cause the destruction of these autoantibodies that blocks the ADAMTS 13 which means you can get better from TTP.

Why is rituximab prescribed?

The use of rituximab has become the main treatment for TTP. During acute episodes, initial treatment with rituximab, plasma exchange, steroids and caplacizumab is now routinely given. Studies have shown that rituximab normalises the abnormal immune system activity. Rituximab may also decrease the frequency of subsequent relapses.

How it is given?

Rituximab is given either in a single or multiple doses by intravenous infusion through a drip in your vein. The type and duration of your treatment will depend on your response to it. This will be determined by your consultant. You will need to attend the haematology day unit for this treatment. The first treatment will take longer, as we will monitor you for any adverse reaction to it. If you are on the multiple dose treatment and no adverse reaction previously, subsequent appointments should not take as long.

What are the possible side effects?

Patients can respond differently to treatment. Not everyone gets side effects, but it is important to be aware if any appear so if you feel unwell or have any of the following signs or symptoms, you can tell your nurse straight away. If you do have a reaction, it can then be treated quickly.

Allergic reaction

This is most likely to happen during the first infusion, so it is given slowly over several hours. Before treatment begins, you will be given medicines to help prevent or reduce any reaction. Allergic reactions can include feeling hot or flushed, developing a skin rash, itching, starting to shiver, feeling dizzy, having a headache, feeling breathless, experiencing swelling of your face or mouth, or pain in your back, stomach or chest.

Risk of infection

This treatment can reduce the number of white blood cells in your blood (neutropenia) meaning you are more likely to get an infection. If you have an infection, it is important to treat it as soon as possible. Speak to your clinical team if you develop unexplained fever (high temperature above 37.5°C) or unexplained low temperature (below 36°C), fatigue (extreme tiredness), sore throat, unexplained diarrhoea (loose stools), ulcers in your mouth or anus, or infection site swelling, pain and rash.

Bruising and bleeding

This treatment can also reduce the number of platelets in your blood (thrombocytopaenia). Tell your doctor if you have any unexplained bruising or bleeding. This may well include nosebleeds, bleeding gums, tiny red or purple spots on the skin that may look like a rash. Some people may need a platelet transfusion, which is given by intravenous drip.

Anaemia

This treatment can reduce the number of red blood cells which carry oxygen around the body (anaemia). If this happens, you may feel tired and breathless. If you are very anaemic, you may need a blood transfusion drip to increase your red blood cells.

Other common side effects

Speak to your healthcare team if you experience any of the following:

• Headaches can be common and can be treated with simple painkillers.
• Swelling or build up of fluid can cause swelling in your limbs, face or other parts of your body.
• Some patients experience hair loss across their body. Your hair will usually grow back once treatment has finished but it is likely to be softer. It may grow back a different colour or be curlier than before.
• Feeling sick (nausea): Feeling or being sick is usually well controlled with anti-sickness medicines which can be prescribed. It might help to avoid fatty or fried foods, eat small meals and snacks and take regular sips of water. Relaxation techniques might also help. It is important to take anti-sickness medicines as prescribed even if you don’t feel sick. It is easier to prevent sickness rather than treat it once it has started.
• Feeling sick (nausea): Feeling or being sick (opens in a new tab) is usually well controlled with anti-sickness medicines which can be prescribed. It might help to avoid fatty or fried foods, eat small meals and snacks and take regular sips of water. Relaxation techniques might also help. It is important to take anti-sickness medicines as prescribed even if you don’t feel sick. It is easier to prevent sickness rather than treat it once it has started.
• Your blood pressure may fall when having rituximab. If you usually take medicine to lower your blood pressure, your doctor may ask you to take this at least 12 hours before having rituximab. Sometimes, rituximab can make your blood pressure go up. Your nurse will regularly monitor your blood pressure to ensure it remains within safe levels.
• Skin changes: This treatment may cause a rash, which can sometimes be itchy. You may also notice unusual feelings in your skin. These include numbness, tingling and burning sensation. Tell your doctor or nurse about any skin changes. They can give you advice and may prescribe creams or medicines to help. Any changes to your skin are usually temporary and improve when treatment finishes.
• Rituximab can interact with other medications including the blood thinning drug warfarin. You should inform your doctor before starting any new medications and you should also tell your GP and other treating clinicians that you are having rituximab.
• Pregnancy and breastfeeding: It is not known whether rituximab would harm an unborn baby or whether it is safe to breastfeed while undergoing treatment. If you are a woman thinking of trying to conceive in the near future, you must take steps to ensure you do not become pregnant while taking rituximab.

Rituximab is safe

Despite the possible side effects, rituximab is safe and well-tolerated by the great majority of patients. Millions of patients worldwide have received rituximab and reports of serious side effects are very rare.

We want you to be fully aware of what this treatment involves and have as much information as possible. If you would like to discuss potential side effects, or have any other concerns, please ask your clinical team. If you agree to have the treatment, we will ask you to sign a consent form.

You will have a physical examination and blood tests prior to treatment and then further monitoring and blood tests afterwards.

Contact information

If you have any questions or concerns, please contact:

Haematology Day Unit: 01223 254590 / 217720 Monday to Friday, 08:00 (8am) to 19:00 (7pm).

Haematology Specialist Nurses Ruth Jolley / Vivian Garcia: 01223 217717

Out of hours, please call the Contact Centre on 01223 245151 and ask to speak to the haematology registrar on call.

Haematology consultants: Dr Martin Besser / Dr Will Thomas / Dr Emily Symington / Dr Jess Griffin / Dr Patrick Sarkies.

Privacy and dignity

Same-sex bays and bathrooms are offered in all wards except critical care and theatre recovery areas where the use of high-tech equipment and/or specialist one-to-one care is required.